Carys Nurse’s big brother smiled as wide as a 10-year-old can when he heard his little sister could get access to life-changing drugs very soon.

“He was shocked when we told him and a big smile came on his face,” said Paul Nurse, eight-year-old Carys’s father.

“When we told her, she sort of shrugged her shoulders,” Nurse said, laughing.

Maybe she was more interested in the monster truck rally the family was heading out to or maybe, at such a tender age, yet another drug to treat her cystic fibrosis hardly seemed something to get excited about.

As for her father and mother, Stacey, they’re over the moon.

Their excitement came with word Friday a pan-Canadian team of negotiators, led by Alberta’s health minister, put together a tentative agreement that could mean an expensive but highly effective drug called Kalydeco could soon be available at a reduced cost to provincial health departments across the country.

“For this sub-group of CF sufferers, it makes all the difference in the world,” he said.

An estimated 100 Canadians have Cystic Fibrosis with a G551D mutation which this drug seems to help. It takes an estimated $300,000 worth of Kalydeco, made by Vertex Pharmaceuticals of Boston, to treat the average sufferer.

Carys was diagnosed with CF when she was only 18 months old. She was started on a regimen of drugs, vitamins, enzyme replacement pills and physiotherapy faced by everyone with this illness.

“Then, in May of last year, we started her on Kalydeco and we couldn’t believe the difference it made in her,” said Nurse.

“Really, it was a close to a cure as we ever thought there could be — she gained weight, she had colour in her cheeks and such an energy level,” recalled Nurse.

Carys was on the drug until December 2013.

“Then my wife lost her job with the federal government,” he said.

That meant a premium health care plan was lost and the family couldn’t afford the medication any longer.

“It broke our hearts — she gets headaches and has lost weight and doesn’t have that high energy level any more,” Nurse added.

Hopefully, it won’t be too long before those symptoms are a thing of the past.

Glavine said there are still details to be ironed out but it’s “pretty much fait accompli.”

“The results have been very good with this drug and it’s a very, very happy day for a lot of people,” Glavine added.

“Tim Vallillee (of Wilmot) has been on it for 23 days and he’s already seeing enormous benefits because he tells me his legs are getting tired before he’s out of breath these days,” Glavine said.

Vallillee is receiving the drug for free, under the drug company’s compassionate program. The Chronicle Herald was not able to contact him for his reaction.

Glavine said at $300,000 per year and as many as seven cases in Nova Scotia, the cost of the drug is really enormous.

“But how do you put a price on such a life-changing drug and, I don’t want to be overzealous, but it may not be too far away when there could be a breakthrough for the rest of those who have CF,” he said.